Harvell J D, Williford P L, White W L
Department of Pathology, Stanford University Medical Center, Stanford, California 94305, USA.
Am J Dermatopathol. 2001 Apr;23(2):116-23. doi: 10.1097/00000372-200104000-00006.
The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.
以下病例报告详细介绍了一名53岁男性，患有良性皮肤或皮肤局限性德戈斯病6年。临床上，患者上躯干和手臂出现弥漫性丘疹。许多丘疹呈现出德戈斯病典型的瓷白色中心，但其他丘疹表现出不同的临床形态，与德戈最初描述的丘疹演变阶段相对应。在几次门诊过程中，患者共接受了5次打孔活检，其组织学表现与临床形态相关。早期丘疹为肤色，表现为浅层和深层血管周围、附件周围及神经周围慢性炎症细胞浸润，并伴有间质粘蛋白沉积。上方表皮显示轻度空泡状界面反应，此早期阶段的组织学表现类似于肿胀性红斑狼疮。完全发育的丘疹隆起，中央有脐凹状瓷白色中心，周围有红斑边缘。组织学上，这些丘疹表现出明显的界面反应，伴有真皮表皮交界处的鳞状化生、色素失禁、表皮萎缩以及乳头真皮硬化的发展区域，类似于微型扁平苔藓硬化萎缩早期阶段。这些界面反应总是局限于打孔活检标本的中央部分，与临床上所见的中央瓷白色区域相对应。完全发育的丘疹的其他特征包括显著的淋巴细胞性血管炎累及小静脉、中性粒细胞和/或嗜酸性粒细胞的轻度附件周围浸润以及间质粘蛋白沉积。在晚期丘疹中，瓷白色区域更明显，病变变平。组织学上，炎症程度通常较轻，整体表现反映了德戈斯病的经典组织学描述，中央有大致楔形的硬化区，上方为萎缩的表皮和角化过度的致密角质层。这些晚期丘疹非常类似于局限于打孔活检标本中心的完全发育的扁平苔藓硬化萎缩的微型版本。
